Pulmonary manifestation of a condition resembling Kasabach–Merritt syndrome in a woman with abdominal angiomatosis associated with consumptive coagulopathy – surgical management: a case report

Journal of Medical Case Reports

Georgios Stamatis²  Dirk Theegarten¹  Stefan Welter²  Thomas Hager¹  Danjouma Housmanou Cheufou² 

[1] Institute of Pathology and Neuropathology, University Hospital of Essen, University of Duisburg-Essen, Essen, Germany;Department of Thoracic Surgery, Ruhrlandklinik-Essen, University of Duisburg-Essen, Essen, Germany

关键词: KMS;    Kasabach–Merritt syndrome;    Hemoptysis;    Abdominal angiomatosis;   

Others  :  1198715
DOI  :  10.1186/s13256-015-0566-z

 received in 2014-07-08, accepted in 2015-03-04,  发布年份 2015

【 摘 要 】

Introduction

Kasabach–Merritt syndrome is a benign condition characterized by hemangiomatosis, severely disseminated intravascular consumption coagulopathy, and thrombocytopenia. The mortality rate increases from 12% to 30% in hemorrhagic cases. In general, the symptoms primarily manifest in the gastrointestinal tract, the skin, and the subcutaneous tissue. There is no publication about pulmonary manifestation of angiomatosis in combination with vascular malformation and hemoptysis. This is the first description of a Kasabach–Merritt syndrome-like condition in the lung.

Case presentation

We present the case of a 29-year-old German woman with angiomatosis and associated pulmonary vascular malformation in her lower left lobe with a Kasabach–Merritt syndrome like condition. It was detected after hemoptysis. We also present our case observations and management.

Conclusion

In a case of angiomatosis with an associated pulmonary lobar vascular malformation, lobectomy can be safely performed to prevent life-threatening bleeding.

【 授权许可】

   
2015 Cheufou et al.; licensee BioMed Central.

【 图 表 】

Figure 1.

Figure 2.

Figure 3.

Figure 4.

【 参考文献 】

• [1]WHO Classification of Tumour of Soft Tissue and Bone. IARC, Lyon; 2013.
• [2]Hall GW. Kasabach-Merritt syndrome: pathogenesis and management. Br J Haematol. 2001; 112:851-62.
• [3]Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Mulliken JB et al.. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pedatr. 2013; 162:142-7.
• [4]Sarkar M, Mulliken JB, Kozakewich HP, Robertson RL, Burrows PE. Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with karposiform hemangioendothelioma and not with common infantile hemangioma. Plast Reconstr Surg. 1997; 100:1377-86.
• [5]Hesselmann S, Micke O, Marquardt T, Baas S, Bramswig JH, Harms E. Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha. Br J Radiol. 2002; 75:180-4.
• [6]Gilon E, Ramot B, Sheba C. Multiple hemangiomata associated with thrombocytopenia: remarks on the pathogenesis of the thrombocytopenia in this syndrome. Blood. 1959; 14:74-9.
• [7]Greinwald JH, Burke DK, Bonthius DJ, Bauman NM, Smith RJ. An update on the treatment of hemangiomas in children with interferon alfa-2a. Arch Otolaryngol Head Neck Surg. 1999; 125:21-7.
• [8]Ferraz AA, Sette MJ, Maia M, Lopes EP, Godoy MM, Petribú AT et al.. Liver transplant for the treatment of giant hepatic hemangioma. Liver Transpl. 2004; 10:1436-7.
• [9]Garcia-Monaco R, Giachetti A, Peralta O, Napoli N, Lobos P, Gioseffi L et al.. Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon: successful treatment with embolization and vincristine in two newborns. J Vasc Interv Radiol. 2012; 23:417-22.
• [10]Wyman LC, Fulton GP, Shulman MH. Direct observations on the circulation in the hamster cheek pouch in adrenal insufficiency and experimental hypercorticalism. Ann N Y Acad Sci. 1956; 56:643-58.
• [11]Bowles U, Kostopoulos-Farri E, Papageorgiou AN. Perinatal hemorrhage associated with the Kasabach-Merritt syndrome. Clin Pediatr. 1981; 20:428-9.
• [12]Fost NC, Esterly NB. Successful treatment of juvenile hemangiomas with prednisone. J Pediatr. 1968; 72:351-7.